Widespread cerebral cortical mineralization in Wilson's disease detected by susceptibility-weighted imaging

Abstract Signal abnormalities of cortical gray matter, compared with the deep nuclear structures, have received less attention in Wilson's disease (WD). They nearly always accompanied white matter signal change, and commonly are associated with epilepsy and psychiatric features. We report herei...

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Bibliographic Details
Published inJournal of the neurological sciences Vol. 313; no. 1; pp. 54 - 56
Main Authors Lee, Jae-Hyeok, Yang, Tae-Il, Cho, Mong, Yoon, Ki-Tae, Baik, Seung-Kug, Han, Yong-Hee
Format Journal Article
LanguageEnglish
Published Amsterdam Elsevier B.V 15.02.2012
Elsevier
Subjects
Adult
Biological and medical sciences
Calcinosis - diagnosis
Calcinosis - metabolism
Calcinosis - pathology
Cerebral Cortex - metabolism
Cerebral Cortex - pathology
Cortex
Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
Diffusion Magnetic Resonance Imaging - methods
Disease Susceptibility - metabolism
Disease Susceptibility - pathology
Hepatolenticular Degeneration - diagnosis
Hepatolenticular Degeneration - metabolism
Hepatolenticular Degeneration - pathology
Humans
Male
Medical sciences
Neurology
Susceptibility-weighted imaging
Wilson's disease
Susceptibility-weighted imaging
Wilson's disease
Cortex
Nervous system diseases
Wilson disease
Central nervous system
Metabolic diseases
Enzymopathy
Nuclear magnetic resonance imaging
Genetic disease
Encephalon
Magnetic susceptibility
Digestive diseases
Copper
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Summary:Abstract Signal abnormalities of cortical gray matter, compared with the deep nuclear structures, have received less attention in Wilson's disease (WD). They nearly always accompanied white matter signal change, and commonly are associated with epilepsy and psychiatric features. We report herein two cases diagnosed as WD who, in addition to characteristic deep nuclear lesions on MR imaging, had widespread cerebral cortical paramagnetic signals dramatically detected by susceptibility-weighted imaging. T2-weighted MR images did not show any cortico-subcortical hyperintense lesions. To our knowledge, these findings have not been described before and may help to further characterize the disease.
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ISSN:0022-510X
1878-5883
DOI:10.1016/j.jns.2011.09.031